Invitrogen™ Bestrophin 1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Brand:  Invitrogen™ PA528910

Product Code. 13473027

Description

Recommended positive controls: NT2D1, PC-3, U87-MG. Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

The retinal pigment epithelium (RPE) and choroid represent a differentiated system of the eye that sustains normal retinal health and function. Best vitelliform macular dystrophy, known as Best disease, is an early-onset autosomal dominant condition in which accumulation of lipofuscin-like material within and beneath the RPE leads to progressive loss of central vision. The lipofuscin-like material in the macular area appears as a yellow mass like the yolk of an egg that later becomes darker and irregular in color, a process known as 'scrambling the egg'. Best disease is frequently a reflection of mutations in the Bestrophin gene, which encodes a protein containing four putative transmembrane domains and localizes to the basolateral plasma membrane of RPE cells. Human Bestrophin forms oligomeric chloride channels that are sensitive to intracellular calcium. Missense mutations at the Bestrophin locus reduces or abolishes Bestrophin protein mediated membrane current. The human Bestrophin gene encodes a 585 amino acid protein.

Specifications

• Antigen: Bestrophin 1
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: BEST1
• Gene Alias: ARB; BEST; BEST 1; Best disease; best macular dystrophy; BEST1; Best1V1Delta2; bestrophin; bestrophin 1; Bestrophin1; Bestrophin-1; BMD; Bmd1; LOW QUALITY PROTEIN: bestrophin-1; mBest1; RP50; TU15B; vitelliform macular dystrophy (Best disease, bestrophin); vitelliform macular dystrophy 2 homolog; vitelliform macular dystrophy protein 2; Vitelliform macular dystrophy protein 2 homolog; VMD 2; Vmd2
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 7439
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Concentration: 1 mg/mL
• Formulation: PBS with 1% BSA, 20% glycerol and 0.01% thimerosal; pH 7
• Gene Accession No.: O76090
• Gene Symbols: BEST1
• Immunogen: Recombinant protein encompassing a sequence within the Intracellular domain of human Bestrophin 1. The exact sequence is proprietary.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG